introitus and vagina must be reconstructed to allow placement of feminine products, including tampons, or even to adequately allow for penetrative intercourse [63]. Utilizing the Female Sexual Function Index, or FSFI, Canalichio etal. found that women with exstrophy scored lower across all domains in the FSFI, including desire, arousal, and lubrication [64]. Though EEC affects males at a rate of 3-to-1, research and improvements in the quality of life for women along the EEC spectrum deserve just as much focus and attention.

The EEC remains one of the most challenging diagnoses for reconstructive sur­geons, owing to its inherent rarity. Add to this the infrequency with which it is seen and operated on, and the complex can easily overwhelm. However, several surgical techniques now exist to allow these children to not only survive infancy but also lead long and productive lives. The MSRE, championed at the authors’ institution, remains the most commonly used method for closure and the technique against which others are compared. It is our hope that, regardless of the technique utilized, careful consideration of the bony pelvis, with a low threshold to perform pelvic osteotomies followed by lower limb immobilization, along with aggressive soft tis­sue mobilization, remains at the forefront of the reconstructive surgeon’s mind and arsenal.

There are several factors involved in the success of managing a patient with blad­der exstrophy, and it is not solely based upon a successful operative closure in the neonatal period, even though we know that children requiring a redo closure fare less well in terms of urinary continence [2]. It is paramount that treatment is under­taken by a bladder exstrophy specialist who has an understanding of the altered anatomy and appreciates the evolution of bladder development, increase in capacity and function throughout childhood and into adulthood. The bladder exstrophy spe­cialist should be part of a bladder exstrophy multi-disciplinary team comprising one

or more urology nurse specialists, psychologist, gynaecologist and orthopaedic spe­cialist. In the United Kingdom, bladder exstrophy care has been centralised to two centres for the last 20years, with the main objective having been to improve out­comes. It is also essential that there exists a pathway for children with bladder exstrophy to transition to an adult or adolescent urologist, and for that individual to possess the same appreciation for the condition and most importantly to have a thorough understanding of the previous surgeries that would have been undertaken.

It is without doubt that Justin Kelly made one of the most noteworthy contribu­tions to the management of children with BEX when he rst described in 1971 ‘a procedure for lengthening the phallus in boys with exstrophy of the bladder’, which we now understand universally as the ‘Kelly procedure’ [3]. It is somewhat ironic that a small spelling mistake in his surname on that publication has kept this paper concealed from those seeking a better understanding of his operation, which still remains somewhat of an enigma if it has not been witnessed it in person. Justin Kelly rst encountered patients with BEX during his fellowship training in Boston where he worked with Robert Gross in the late 1960s [4]. He encountered many patients with disappointing clinical outcomes whom at the time were undergoing a cystectomy and ureterosigmoidostomy. Although they were continent of urine, it was evident that the older male patients with BEX were psychologically scarred by the short penile length and reportedly some males were committing suicide [4]. Justin Kelly undertook a detailed anatomical dissection of the male genitalia in the autopsy room and conceptualised a new technique that would enhance and ‘nor­malise’ the anatomy. He initially proposed the procedure to lengthen the penis in male patients with bladder exstrophy [3]. He described the identication and mobil­isation of the neurovascular pedicle within Alcock’s canal that supplied each of the corporal bodies and a method for detaching the corporal bodies from their attach­ment to the ischiopubic ramus (Fig.8.1). This manoeuvre would allow the corporal bodies to come into approximation and move out of the pelvis, thereby enhancing the visibility of the penis. Over time Justin Kelly modied and further rened the technique, both when he returned to Melbourne, Australia, and subsequently at Great Ormond Street Hospital for Children, London. By the time he was working in London, the Kelly procedure had already matured from a penile lengthening to a continence procedure, by virtue of the fact that the exposure achieved by the radical dissection allowed an orthotopic reconstruction of the bladder neck and an external urethral sphincter mechanism.

The bladder should be separated from the rectus muscle, taking care not to dam­age the detrusor muscle. The dissection should continue inferiorly towards the tri­gone and area of the verumontanum, until the point is reached where the attachment of the corpora to the ischiopubic ramus is visualised. It is essential to preserve an adequate amount of detrusor muscle in the region of, and proximal to, the verumon­tanum, as this will be utilised for the placement of the sutures in the construction of the bladder neck.